Imported cutaneous leishmaniasis caused by Leishmania major in a Chinese laborer who worked in Saudi Arabia 655
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Milia-like calcinosis cutis in a girl with Down syndrome
*Berna Solak
1Rabia Oztas Kara
1Erdem Vargol
2s
Received on 15.03.2015. Approved by the Advisory Board and accepted for publication on 20.06.2015. * Work conducted at the School of Medicine of Sakarya University, Sakarya, Turkey.Financial Support: None. Conflict of Interest: None. 1 Sakarya University, School of Medicine – Sakarya, Turkey. 2 Sakarya University Training and Research Hospital – Sakarya, Turkey. ©2016 by Anais Brasileiros de Dermatologia DOI: http://dx.doi.org/10.1590/abd1806-4841.20164560 Abstract: Milia-like idiopathic calcinosis cutis (MICC) is a very rare dermatological disorder characterized by multiple whitish to skin colored, milia-like papules, mostly found on the hands. MICC can disappear spontaneously by adulthood; therefore, its early recognition is crucial to avoiding unnecessary interventions. Herein, we present a case of MICC in a 6-year-old girl with Down syndrome.
Keywords: Calcinosis; Molluscum contagiosum; Skin diseases
INTRODUCTION
Milia-like idiopathic calcinosis cutis (MICC) is a very rare dermatological disorder characterized by multiple whitish to skin colored, firm, tiny milia-like papules, mostly on the hands and feet. MICC was first reported in 1978.1,2 Since then, to the best of our knowledge, only about 25 cases have been reported in English language medical literature, mostly in association with Down Syn-drome.1 Herein, we present this rare case study to help the busy dermatologist or pediatrician to recognize this disorder in his/her differential diagnoses of milia-like lesions. CASE REPORT A 6-year-old girl was received at our medical center com-plaining of multiple asymptomatic, 1–2 mm in diameter, whitish papules on her hands, feet, and face. The patient reported that the papules on her hands and feet had been present for three years, while those on her face had first appeared last year, and that these lesions were increasing (Figures 1A and 1B). Her mother denied any
previous trauma or wounds at the sites of the lesions. The patient presented trisomy 21 (Down syndrome), hypothyroidism, grade III vesicoureteral reflux, as well as atrial and ventricular septal defects. As treatment, she was taking levothyroxsine (Levotiron®
). Dermo-scopic appearance did not show typical changes associated with MICC (Figure 1C). Skin biopsies of the lesions revealed an accumu- lation of well-defined, dense, basophilic material surrounded by fi- brous tissue in the dermis (Figure 1D). Biochemical studies, includ-ing serum calcium, phosphate, and parathyroid hormone levels, were within the normal range. Based on pathological and clinical findings, the patient was diagnosed with MICC.
DISCUSSION
MICC is a form of idiopathic calcinosis cutis, which implies an abnormal deposition of insoluble calcium salts in the dermis. MICC’s exact etiology and pathogenesis is unknown; however, se- rum calcium and phosphorus levels do remain normal, unlike met-655
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astatic or iatrogenic calcinosis. 3,4 This disorder is equally frequent in
both sexes and most commonly occurs during childhood.1 Though
lesions are frequently found on the hands and feet, they may also appear on other parts of the body. MICC normally disappears spon-taneously by adulthood, mostly without scarring, 4 and has mostly
been reported as associated with Down syndrome.1 Although the
pathogenesis of MICC is unclear, medical studies have shown that patients with Down syndrome have ectopic calcifications, such as valvular and basal ganglial calcifications.5,6 MICC has also been
re-ported to occur in patients without Down syndrome. It can there-fore be suggested that other unknown factors may play important roles in its pathogenesis. Although not proven clearly, some authors have suggested that sweat glands may play a role in calcium depo-sition by means of decreased excretion or increased sweat calcium concentrations.7,8
Moreover, it is thought that recurrent, minor trau-mas may in fact trigger this disorder. 9
Distinguishing MICC from molluscum, milium, verruca, xanthomas, or inclusion cysts is essential. Diagnosis is based on his-topathological, clinical, and laboratory findings. Histopathological examination reveals foci of a well-circumscribed, round, basophilic 656 Solak B, Kara RO, Vargol E
FIgure 1: a.
Whitish papules on the face (red arrow); B. Whit-ish papules on the foot ; C. Der-moscopic appear-ance of the lesion (black arrow); D. A c c u m u l a t i o n of well-defined, dense, baso-philic material surrounded by fibrous tissue in the dermis (100x H&E) A C B D substance, which is stained black with Von Kossa stain, in the upper dermis, surrounded by thick collagen fibers, and sometimes, epithe-lioid and multinucleated giant cells.4,9
Dermoscopy is commonly proposed as a helpful method for differential diagnosis.10 Recently, Fox et al.1, upon dermoscopic
examination, described a subtle petaloid appearance, though not pathognomonic for MICC.The dermoscopic examination of our pa-tient’s lesions proved unremarkable, demonstrating a homogenous, bright white, round area which could not be distinguished from mil-ium or molluscum.
Since this disorder can disappear spontaneously by adult-hood, unnecessary aggressive interventions are not recommended. On the other hand, tretinoin cream was found useful by Choi et al. 4
Because of the possibility of spontaneous recovery, watchful follow-up may be the best approach. Since diseases in the differ-ential diagnosis of MICC, i.e. molluscum, verruca, and milium, are usually treated with traumatic methods, such as cryotherapy and cauterization, it is important to distinguish MICC from these disor-ders, as these therapeutic modalities may serve only to worsen the lesions and stimulate recurrence. q
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